ALS_rad.jpg SHOWN: Dr. Rick Olney does physical therapy in the Neurofit room at UCSF to hold off the effects of his ALS. He's watched lovingly by his son Nick Olney. Story about ALS, Amyotrophic Lateral Sclerosis, (Lou Gehrig's Disease). We interview Dr. Rick Olney at UCSF whose research has focused on this disease. Carl Hall is the reporter. Katy Raddatz / The Chronicle MANDATORY CREDIT FOR PHOTOG AND SF CHRONICLE/ -MAGS OUT #MainNews#Chronicle#11/29/2004####0422460753 #MainNews#Chronicle#11/29/2004####0422460753 #MainNews#Chronicle#11/29/2004####0422460753

Dr. Richard Olney first got interested in the neuromuscular disease known as amyotrophic lateral sclerosis, or ALS, when the daughter of his favorite teacher in junior high school died of it.

He soon came to regard ALS, also known as Lou Gehrig's disease, as one of the most compelling -- and justly feared -- disorders of the nervous system. And so, during 25 years as a neurologist, Olney devoted his career to the care of ALS patients, founding a specialized clinic at UCSF.

He resigned as clinic director this summer and has stopped treating all his patients with ALS. But he has even more reason these days to be interested in their disease.

Now, it is his disease.

In June, Olney, 56, regarded as one of the top ALS clinicians in the country, was diagnosed with ALS.

ALS is not contagious. He didn't catch it from close contact with a patient. It's just a matter of unhappy fortune -- just one more case in one of the biggest ongoing mysteries of brain research.

A familial type accounts for a small number of cases, but for the most part ALS strikes without any warning or previous family history of the disease. "Ninety to 95 percent of cases just happen at random," Olney said.

The diagnosis has shocked hundreds of ALS patients and family members throughout the West who have received care and counseling from Olney at the UCSF clinic, one of only two in the state and 19 in the country certified as a regional center by the ALS Association.

Amid an outpouring of sympathy, the tragedy has focused new attention on efforts to find a cause and eventually a cure for the invariably fatal disease.

UCSF has scheduled a research symposium in Olney's honor on Dec. 17, including presentations by two university colleagues and collaborators, Nobel laureate Dr. Stanley Prusiner and stem-cell specialist Dr. Arnold Kriegstein.

Although Olney has concentrated mainly on patient care, he also has emphasized the need for more research, participating in a number of national multi-center trials. "We share a fraternity," said Dr. Jeffrey Rothstein, a neurology professor and ALS researcher at Johns Hopkins University, who also is scheduled to speak at the symposium. "Rick is a highly respected clinical investigator in the field of ALS. It's a bad enough disease, but for one of your own colleagues to develop the same disease you're studying. ... Well, I've never heard of it happening."

Lifespan measured in years -- or months

People with ALS typically expect to live about two or three years, perhaps as long as five years, after diagnosis. Olney's case does not appear to be the slow-moving kind. He may have only a matter of months.

That's according to Dr. Cathy Lomen-Hoerth, a UCSF neurologist, who took over at the ALS clinic when Olney found he no longer could stand up, let alone muster the strength to conduct neurological exams.

For years, Lomen-Hoerth was Olney's medical protege. She is his doctor now.

When advising her patient, Lomen-Hoerth said she often gets the sense of hearing echoes of his own teaching, which emphasized a comprehensive approach that may include detailed, hours-long examinations and close consultation with a diverse team of medical specialists.

"It still is hard to believe, even now," she said. "You see him getting worse and so you know it's true. But it's so hard to believe he could actually get this disease that he has worked so hard to impact over the years."

Olney, interviewed in his former office and while undergoing his therapeutic-exercise routine at the hospital, was more matter-of-fact about it.

He knows the risk numbers for ALS as well as anybody: The lifetime cumulative risk is about one case per 1,000 people, about the same as multiple sclerosis. Only about 30,000 people in the United States have ALS, compared with about 10 times that number with MS, but that's mainly because people with MS live a lot longer.

"ALS is really not as rare as you might think," Olney said.

Given the odds, Olney calculates there should be a case once every five or 10 years among those working on the disease. So he figures a case like his was overdue. He said he just had a hard time being convinced that he was the one, saying it just seemed too ironic.

At first, Olney suspected the instability he began noticing in his right knee, and later a progressive weakening in both legs, was caused by compression of a disk in his spinal column. A longtime runner, he thought it might be related to muscle damage he sustained while training for his fourth marathon.

But after three surgeries failed to halt the early symptoms, Dr. Robert Layzer, Olney's colleague in the UCSF neurology department, confirmed that the culprit was ALS.

Despite the surgeries, the weakness spread to his arms. Lately it's noticeably affected his speech. It will, in all likelihood, keep getting worse, eventually making it difficult even to swallow, or breathe. Then it will end.

ALS is marked by the degeneration of select nerve cells, originating in the brain or spinal cord, that drive the muscles. One of its more unsettling characteristics is that it spares most parts of the brain, including the cortical neurons that underlie alertness and conscious thought. So patients get a front-row seat at their own demise.

The root cause is unknown. No one has found a way to fundamentally alter the course of the disease. The best that can be achieved from drug treatments, Lomen-Hoerth said, is to slow it down, and not by much.

Research now focuses on ways of shoring up the nervous system's defenses, perhaps by using gene therapy to deliver a nerve growth factor precisely where it's needed. Lomen-Hoerth, among others, is trying to root out the genetic and environmental factors that make some people susceptible to ALS, or may cause some patients to decline more quickly than others.

"We need a broader, more comprehensive understanding of this," said Graeme Davis, a UCSF biochemist working on the problem. "We're still at kind of the basic level now, but the exciting thing is that as more gene candidates are found these days, the knowledge gap closes."

'A degree of insight'

It's not likely to close quickly enough to change the outcome for the UCSF neurologist. Olney said his long experience working with ALS patients has helped.

"It gives me a degree of insight into some of the practical details, how this affects your life at home and in the community," he said, noting he wasted no time adding the wheelchair ramps and other equipment he knew he and his caregivers would need at his home.

He said his medical training has helped him face death squarely.

"You have to deal with your own mortality to really be comfortable dealing with dying people," he said. "So that's made it a lot easier for me."

His wife, Paula, a dialysis nurse, said it has been harder for her.

"I've been so emotional, I've been crying for a whole year," she said.

Olney said he never forgot an encounter early in his career with a young man dying of stomach cancer, who had the discipline to allow himself but a half hour every morning of teary emotion, and then devoted the rest of the day to enjoying life as best he could.

That's become the strategy in the Olney household, too. The couple's two adult children, Nick and Amy, have moved back to their Marin home, each taking a turn caring for their father. Paula Olney said he still orchestrates everything, adding that they have had much to do, coping with the endless details involved in "shutting down a career and a life."

"He's holding up all of us," Paula Olney said. "His disease is progressing surprisingly fast but he is still positive. He's not angry. He doesn't sit there like a depressed guy."

Professional colleagues have been most impressed by Olney's determination to continue contributing to ALS research, not as a doctor now but as a patient willing to submit to placebo-controlled clinical trials. That means he may be given a sugar pill, rather than an actual drug.

Shortly before his forced retirement, Olney wrote a protocol for such a clinical trial, a complicated effort that will test two experimental drugs at the same time, head to head and versus a placebo. Lomen-Hoerth now plans to direct the effort, and will soon begin enrolling patients.